胰腺背侧完全发育不全——罕见的先天性异常:病例表现、影像学表现及文献回顾

Ravinder Kumar, K. Vyas, N. Agrahari, J. Kundu
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引用次数: 6

摘要

胰腺背侧完全发育不全是一种罕见的先天性异常,因为胰腺解剖变异的频率很低。由于这种情况极为罕见,在世界文献中报道的病例不到100例。我们在此报告一个三十五岁男性诊断为胰腺背侧发育不全(ADP)的病例,并表现出重度酒精滥用引起的糖尿病和急性胰腺炎的附加特征。生化评价显示血清淀粉酶和胰脂肪酶升高(分别为516 U/L和912U/L);正常值0 ~ 200和0 ~ 190)。腹部超声显示胰腺体和胰腺尾未见。腹部CT及MRCP示胰腺颈部、体部、尾部及圣托里尼管未见,十二指肠小乳头未见。这三种诊断证实了ADP的诊断。本病例报告是关于ADP的放射学表现,相关症状和管理的描述,在相关的世界文献。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Complete Agenesis of Dorsal Pancreas -A Rare Congenital Anomaly: Case Presentation with Imaging Findings and Review of Literature
Complete agenesis of the dorsal pancreas is a rare congenital anomaly due to low frequency of anatomic variations in pancreas. Due to this exceedingly rare occurrence, less than 100 cases have been reported in world literature. We report here a case presentation of thirty five year old male diagnosed with Agenesis of Dorsal Pancreas (ADP) presenting with additional features of diabetes mellitus and acute pancreatitis from heavy alcohol abuse. Biochemical evaluation showed raised serum amylase and serum pancreatic lipase (516 U/L and 912U/L; normal values 0-200 and 0-190, respectively). Ultrasound abdomen exhibited absence of body and tail of pancreas. CT abdomen and MRCP revealed absence of neck, body, and tail of the pancreas along with duct of Santorini, and the minor duodenal papilla. This diagnostic triad confirmed the diagnosis of ADP. This case report is concerned with the description of radiological appearances of ADP, associated symptoms and management in pertinent light of world literature.
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